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Thoracic Aorta Disease- Under Review

Checked: 23-05-2022 by Rob Adams Next Review: 23-05-2023

Overview

This page deals with management of patients with:

Imaging that indicates dilated ascending/thoracic aorta who are asymptomatic
Patients who have a strong family history of dilated thoracic aorta or dissection.

See the following pages for the other conditions:

Thoracic aortic disease is most commonly cause by a combination of age and hypertension. A significant minority of cases are inherited (syndromic and non-syndromic), and known as hereditary thoracic aortic disease (HTAD).

The increase in availability of genetic testing has resulted in increased referrals to the service and the need for guidance for referral, genetic testing, and appropriate follow-up intervals.

A dilated ascending aorta may be found as an incidental finding on imaging such as echo or MRI or during investigation of associated conditions such as:

If the patient is symptomatic then please review the Aortic Dissection page or see the Red Flag section below.

Family History

About 20% of patients with thoracic aortic aneurysm or dissection (TAAD) have a first-degree relative with a similar disease. The familial form (FTAAD) of the disease is inherited in an autosomal-dominant pattern. Current guidelines for thoracic aortic disease recommend screening of first-degree relatives of TAAD patients. In known familial disease, screening of both first- and second-degree relatives is recommended(1).

Who to refer

If asymptomatic then patients should be referred routinely to Cardiology via eRS to discuss monitoring if any of the following apply:

Family history aortic or vessel aneurysm/dissection
Bicuspid aortic valve
Aorta >40mm

The patient should also be suitable for surgery - this may not be appropriate if the patient is frail or not willing to consider surgery.

Local criteria for referral are currently being developed and will be published here when available.

In addition, all patients with a first degree relative with a dilated thoracic aorta should also be referred for screening. Patients with known familial disease in a first or second degree relative should also be referred for screening. There is currently no formal pathway for screening in BNSSG, so for now please request Cardiology advice and guidance from your nearest hospital initially to request advice on appropriate management.

Red flags

Suspected aortic dissection

Any person with new onset pain who has a history of aortopathy (or diagnosed first degree relative), aortic dilatation, bicuspid aortic valve or with past aortic surgery should have an emergency CT aortogram to exclude aortic dissection.

What to do before referral

For patients with asymptomatic dilatation of the ascending aorta, please consider the following as part of the work up:

Family History
Blood pressure
ECG
CXR
Echocardiogram

All patients should also have cardiovascular risks managed as appropriate.

Safety netting - advise the patient that if they develop chest pain then they should call 999 and attend their local emergency department immediately.

Referral

UHBW - Refer to Cardiology - Valve Clinic at the BRI via eRS.

NBT - Refer to cardiology via eRS.

GPs may also consider using Cardiology advice and guidance which is available via eRS.

Resources

(1) Screening for Familial Thoracic Aortic Aneurysms with Aortic Imaging Does Not Detect All Potential Carriers of the Disease - PMC (nih.gov)

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

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